Sickle Cell Disease
Conditions
Keywords
Sickle Cell Disease, Echocardiography, Speckle tracking
Brief summary
Sickle Cell Disease is a serious disease that is life-threatening for patients being homozygous for the SS form or heterozygous for the SC or βthal forms. The CHU Brugmann hospital currently regularly treats about 70 homozygous adult patients and this number is in constant augmentation. Sickle cell disease patients may develop a cardiomyopathy due to chronic anemia, the haemosiderosis risk or, less frequently, to coronary vaso-occlusive damages. The hypervolemia in patients with sickle cell disease causes an overestimation of the ejected left ventricular fraction measured by echocardiography, this parameter being very dependent of the blood volume.It has already been shown that the left ventricular ejection fraction was normal in most patients with sickle cell disease, but that its evaluation by parameters independent from the blood volume showed the existence of a dysfunction. Myocardial strain, as measured by speckle tracking, is a echographic evaluation method of the cardiac function, independent of the blood volume. This technique hasn't been used much in sickle cell disease patients. A study using 3D speckle tracking on a limited number of sickle cell disease patients failed to show a strain anomaly. Moreover, the study highlighted a higher global longitudinal strain in this patient population. The investigators find these data hard to explain and in contradiction with previous studies using other cardiac function evaluation techniques, independent from the blood volume. The primary goal of this study is thus * to study the longitudinal strain by 2D echography * to determine if anomalies of the longitudinal strain exist in sickle cell disease patients with a normal ejected left ventricular fraction, compared to a control group of healthy patients. The secondary goal of this study is to correlate, inside the sickle cell disease group, the possible strain anomalies with biological gravity parameters of the disease.
Interventions
OTHERCardiac echography
Ejection fraction measured by Teicholz and planimetry, diastolic function, tissular doppler, myocardiac performance index, global longitudinal strain measured by speckle tracking, arterial pulmonary hypertension, left ventricular hypertrophy.
Hemoglobin levels, red cells, hematocrit, iron, ferritin
OTHERClinical parameters
Blood transfusion number, severity of the sickle cell disease damage, evolution duration of the sickness
Sponsors
Brugmann University Hospital
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* All sickle cell disease patients
Exclusion criteria
* Insufficient echogenicity
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Cardiac diastolic function | once per year, at the annual medical visit planned according to the standart of care for this pathology | — |
| Cardiac tissular doppler | once per year, at the annual medical visit planned according to the standart of care for this pathology | — |
| Myocardiac performance index | once per year, at the annual medical visit planned according to the standart of care for this pathology | — |
| Global longitudinal strain | once per year, at the annual medical visit planned according to the standart of care for this pathology | Global longitudinal strain measured by speckle tracking. |
| arterial pulmonary hypertension | once per year, at the annual medical visit planned according to the standart of care for this pathology | — |
| left ventricular hypertrophy | once per year, at the annual medical visit planned according to the standart of care for this pathology | — |
| Cardiac ejection fraction | once per year, at the annual medical visit planned according to the standart of care for this pathology | Ejection fraction measured by Teicholz and planimety. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Biological parameters: hemoglobin levels | once per year, at the annual medical visit planned according to the standart of care for this pathology | — |
| Biological parameters: ferritin levels | once per year, at the annual medical visit planned according to the standart of care for this pathology | — |
| Biological parameters: red cells count | once per year, at the annual medical visit planned according to the standart of care for this pathology | — |
| Biological parameters: hematocrit levels | once per year, at the annual medical visit planned according to the standart of care for this pathology | — |
| Biological parameters: iron levels | once per year, at the annual medical visit planned according to the standart of care for this pathology | — |
| Clinical parameters: severity of the illness | once per year, at the annual medical visit planned according to the standart of care for this pathology | Sickle cell disease organ damages. |
| Clinical parameters: sanguine transfusion numbers | once per year, at the annual medical visit planned according to the standart of care for this pathology | — |
Countries
Belgium
Outcome results
None listed